The Scimitar Syndrome in infants and children

pp 180-184

Abstract

Background

The scimitar syndrome (SS) has a variable clinical profile based on age of presentation. 

Objective

To compare the different clinical aspects and treatment between infants younger and older than one year.

Material and methods

From 1997 to 2003, SS was diagnosed in 14 consecutive patients which were divided into two groups. Group I: 9 patients, mean age 2 months (range 2 days -7 months) ando Group II: 5 patients mean age 4.8 years (range 1-12.7 years). The presence of heart failure (HF), pulmonary hypertension (PH), systemic collateral embolization, surgical treatment and clinical outcome were assessed. 

Results

Nine patients were admitted with HF, all belonging to group I (p=0.012). PH was present in 7 patients; Group I: 7/9 and group II: 0/5 (p=0.005). Twenty systemic collateral embolizations were performed; Group I: 17 procedures in 9/9, 5 of them needed two or more embolizations due to re-permeabilization of the collateral, Group II: 3 embolizations in 2/5 patients (p=0.008). Eleven patients underwent surgical repair; Group I: 8/9 and Group II: 3/5 (p=0.52). One patient in Group I, who had several collateral embolizations and had indication for surgery, died because of PH and HF. 

Conclusion

Patients younger than one year had a higher incidence of HF, PH and collaterals, requiring larger number of systemic collateral embolizations. Patients older than than one year had better clinical outcome.